Von Willebrand Disease

Question 1

What is Von Willebrand Disease?

Answer 1

An inherited bleeding disorder that results in increased bleeding risk

Question 2

What is the function of vWF?

Answer 2

Von willebrand factor creates cross links between platelets and the endothelium of blood vessels. It also stabilised factor VIII

Question 3

What is the aetiology of von willebrand disease?

Answer 3

A mutation in the vWF gene leads to decreased quantity or function of vWF

Question 4

What are the types of von willebrand disease?

Answer 4

Type 1 - reduced quantity
Type 2 - Abnormal vWF
Type 3 - complete lack of vWF

Question 5

What is the presentation of von willebrand disease?

Answer 5

Excessive bleeding from minor wounds
Post-operative bleeding
Heavy menstrual bleeding
Easy bruising

Question 6

What investigations are helpful in the diagnosis of von willebrand disease?

Answer 6

APTT
PTT
FBC
vWF antigen
Factor VIII activity

Question 7

What blood results will be seen in von willebrand disease?

Answer 7

Normal PTT
Increased APTT
Prolonged bleeding time
Reduced factor VIII

Question 8

What is the first line management of von willebrand disease?

Answer 8

vWF factor and factor VIII concentrates
Desmopressin
Tranexamic acid

Question 9

What is the function of desmopressin for von willebrand disease?

Answer 9

Increases levels of vWF in the blood by allowing the release of vWF from Weibel-Palade bodies in endothelial cells